Extent of lung fibrosis is of greater prognostic importance than HRCT pattern in patients with progressive pulmonary fibrosis: data from the ILD-PRO registry

作者：Aparna Swaminathan, Jeremy M. Weber, Jamie L. Todd, Scott M. Palmer, Megan L. Neely, Timothy P. Whelan, Grace Hyun J. Kim, Thomas B. Leonard, Jonathan Goldin · 发表于：Respiratory Research · 年份：2025 · DOI：10.1186/s12931-025-03136-6 · 被引用次数：6 · 研究领域：Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Systemic Sclerosis and Related Diseases、Chronic Obstructive Pulmonary Disease (COPD) Research

BACKGROUND: The prognostic value of patterns and quantitative measures of lung fibrosis on high-resolution computed tomography (HRCT) in patients identified as having progressive pulmonary fibrosis (PPF) has not been established. We investigated whether HRCT patterns and quantitative scores were associated with risk of progression in patients with PPF. METHODS: Patients enrolled in the ILD-PRO Registry had an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis, reticular abnormality and traction bronchiectasis, and met criteria for ILD progression. HRCT images taken between 24 months prior to enrollment and 90 days after enrollment were analyzed using a machine learning algorithm to derive quantitative scores. Associations were assessed between HRCT pattern (usual interstitial pneumonia [UIP]-like versus other patterns) and tertiles of quantitative scores and measures of disease severity at enrollment, and between these patterns/tertiles at enrollment and ILD progression (relative decline in forced vital capacity [FVC] % predicted ≥ 10%, lung transplant, or death) over a median follow-up of 17.3 months. RESULTS: Among 395 patients, 178 (45.1%) had a UIP-like pattern on HRCT. A UIP-like pattern did not associate with worse disease severity at enrollment or an increased risk of ILD progression (HR 1.01 [95% CI: 0.71, 1.44]). The highest quantitative lung fibrosis (QLF) score tertile (≥ 20.5%) was associated with worse disease severity. In unadjusted analyse...